Article
Rheumatology
Jerome Hadjadj, Alice Canzian, Omer Karadag, Anne Contis, Francois Maurier, Sebastien Sanges, Silvia Sartorelli, Laure Denis, Claire de Moreuil, Cecile-Audrey Durel, Stephane Durupt, Marie Jachiet, Diane Rouzaud, Carlo Salvarani, Roberto Padoan, Lorenzo Dagna, Fabrice Bonnet, Christian Agard, Thomas Moulinet, Marion Hermet, Raluca Sterpu, Alexandre Thibault Jacques Maria, Jeremy Keraen, Loic Guillevin, David Jayne, Benjamin Terrier
Summary: This study investigates the effectiveness and safety of biologics, particularly TCZ, in treating relapsing and/or refractory polyarteritis nodosa (PAN) patients. The results suggest that TCZ may be effective in managing PAN, but further research is needed to confirm these findings.
Review
Rheumatology
Eleni Papachristodoulou, Loukas Kakoullis, Eleni Tiniakou, Konstantinos Parperis
Summary: The study reviewed different treatment modalities for Cutaneous polyarteritis nodosa (CPAN) and found limited evidence on the management of CPAN patients. Current treatment mainly involves the use of systemic corticosteroids and immunosuppressive agents in combination, but further research is needed to evaluate their impact on disease remission, relapses, and mortality.
Review
Biochemistry & Molecular Biology
Louis Wolff, Alice Horisberger, Laura Moi, Maria P. Karampetsou, Denis Comte
Summary: Polyarteritis nodosa is a form of necrotizing vasculitis that primarily affects medium-sized vessels, but can also involve smaller vessels. It has a wide range of clinical manifestations, with common symptoms including shortness of breath, fever, and weight loss. The skin, nervous system, and gastrointestinal tract are frequently affected. While it was historically linked to hepatitis B virus, recent research has found associations with genetic syndromes and neoplasms.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Rheumatology
Simon Carette
Summary: The author reflects on their medical school journey from 50 years ago and plans to give a lecture in 2022 to review the knowledge of vasculitis before 1971 and what has been learned since.
JOURNAL OF RHEUMATOLOGY
(2022)
Article
Dermatology
Athanassios Kolivras, Curtis Thompson
Summary: Macular arteritis (MA) is characterized by a discordance between clinical presentation of hyperpigmented macules and histopathologic findings of lymphocytic arteritis, primarily affecting middle-aged women's legs, sometimes with a more severe variant. Despite similar histologic features, there is no evidence of progression from MA to lymphocytic thrombophilic arteritis in terms of disease course.
CLINICS IN DERMATOLOGY
(2021)
Article
Rheumatology
Theresa Graalmann, Katharina Borst, Himanshu Manchanda, Lea Vaas, Matthias Bruhn, Lukas Graalmann, Mario Koster, Murielle Verboom, Michael Hallensleben, Carlos Alberto Guzman, Gerd Sutter, Reinhold E. Schmidt, Torsten Witte, Ulrich Kalinke
Summary: Rituximab-treated patients and B cell-deficient mice showed reduced expansion of virus-specific CD8(+) T cells after vaccination/infection with different vaccines/pathogens, indicating that B cells can modulate CD8(+) T cell responses. The choice of vaccines for B cell-depleted patients needs to be re-evaluated to effectively induce protective CD8(+) T cell responses.
ANNALS OF THE RHEUMATIC DISEASES
(2021)
Review
Cardiac & Cardiovascular Systems
Hong Huang, Yanjun Gong, Li Guo, Zhuoli Zhang
Summary: This case report presents a 22-year-old male patient with polyarteritis nodosa (PAN) who developed insidious occlusion of three main coronary arteries after 7 years of stable PAN. Treatment with a stent implantation and anti-angina medications alleviated the chest pain.
BMC CARDIOVASCULAR DISORDERS
(2021)
Article
Medicine, General & Internal
Kerilyn Godbe, Ashlie Elver, Peter Chow, Chris Williams, Garth Fraga, Penelope Harris, Mohammed Taha, Dhaval Bhavsar, Richard Korentager
Summary: This report describes a case of lower extremity ulcers in a female patient following COVID vaccination. Despite various treatments, the ulcers continued to progress. Rare vascular and subcutaneous calcifications may be related to calciphylaxis. This case highlights the challenges of managing rare diseases and the importance of multidisciplinary cooperation.
FRONTIERS IN MEDICINE
(2022)
Article
Cardiac & Cardiovascular Systems
David Saadoun, Mathieu Vautier, Patrice Cacoub
Summary: Systemic vasculitides are a type of multisystem blood vessel disorders classified by vessel size. Prompt identification is crucial due to the potentially serious outcomes associated with these conditions. Ethnic and regional differences in incidence and clinical characteristics should be taken into account.
Article
Immunology
Julien Rohmer, Yann Nguyen, Ludovic Trefond, Christian Agard, Jean Sebastien Allain, Alice Berezne, Pierre Charles, Pascal Cohen, Guillaume Gondran, Matthieu Groh, Tessa Huscenot, Carole Lacout, Estibaliz Lazaro, Jonathan London, Francois Maurier, Arsene Mekinian, Rafik Mesbah, Isabelle Nubourgh, Laurent Perard, Xavier Puechal, Gregory Pugnet, Mathieu Puyade, Viviane Queyrel, Arthur Rouxw, Diane Rouzaud, Cecile-Audrey Durel, Loic Guillevin, Benjamin Terrier
Summary: The landscape of PAN has changed in the last decades, with the disappearance of HBV-related PAN and the emergence of secondary PAN. The relapse rate remains high, especially in older patients with gastrointestinal and skin necrosis. The 1-, 5-, and 10-year overall survival rates were 93%, 87%, and 81%, respectively.
JOURNAL OF AUTOIMMUNITY
(2023)
Article
Rheumatology
Alexandre Fagart, Thomas Machet, Guillaume Collet, Thomas Quemeneur, Rim Ben Ticha, Marion Verstraete, Noemie Le Gouellec, Franck Demailly, Clementine Rousselin
Summary: This study aimed to describe the findings of FDG-PET/CT in patients with newly diagnosed PAN. The results showed that FDG-PET/CT seems to be a useful non-invasive imaging technique for diagnosing PAN, particularly in patients with non-specific systemic features. Increased tracer uptake in muscular connective tissue appears to be a common and potentially pathognomonic feature in patients with PAN.
Review
Rheumatology
Shun Nomura, Yasuhiro Shimojima, Yasufumi Kondo, Dai Kishida, Yoshiki Sekijima
Summary: HP is a rare neurological disorder characterized by thickening of the dura mater, commonly leading to symptoms such as headache and cranial neuropathies. This case study presented a patient with HP following a diagnosis of PAN, who showed improvement with corticosteroid and cyclophosphamide treatment. Subsequent maintenance therapy with methotrexate helped maintain remission and reduce prednisolone dosage. Only two cases of HP in PAN patients have been reported in English literature, indicating its rarity as a complication of PAN.
CLINICAL RHEUMATOLOGY
(2022)
Article
Rheumatology
Samuel Bitoun, Julien Henry, Delphine Desjardins, Christelle Vauloup-Fellous, Nicolas Dib, Rakiba Belkhir, Lina Mouna, Candie Joly, Marie Bitu, Bineta Ly, Juliette Pascaud, Raphaele Seror, Anne-Marie Roque Afonso, Roger Le Grand, Xavier Mariette
Summary: The antibody response to the mRNA COVID-19 vaccine is diminished in patients treated with rituximab (RTX), but the functional T cell response remains unchanged compared to patients treated with other immunosuppressants and healthy controls.
ARTHRITIS & RHEUMATOLOGY
(2022)
Article
Medicine, General & Internal
In Jun Han, Chang Hyeon Jeong, Hyoseon Choi
Summary: This case report presents a patient initially presenting with peripheral neuropathy and myositis without typical organ involvement, but was ultimately diagnosed with polyarteritis nodosa (PAN). The case reminds us to consider the potential for PAN in patients presenting with radicular and focal muscle pain.
Article
Immunology
Martina Fabris, Ginevra De Marchi, Rossana Domenis, Federica Caponnetto, Silvia Guella, Chiara Dal Secco, Nicola Cabas, Salvatore De Vita, Antonio Paolo Beltrami, Francesco Curcio, Luca Quartuccio
Summary: This study evaluated the immune response to SARS-CoV-2 mRNA vaccination in patients with complex or rare systemic autoimmune diseases who were previously treated with or under continuous treatment with B-cell-targeted therapies. The results showed that even in the absence of circulating B cells, virus-specific cellular immunity can be induced, indicating that B-cell-targeted therapies do not preclude SARS-CoV-2 vaccination.
JOURNAL OF AUTOIMMUNITY
(2022)
Article
Rheumatology
Masataka Kuwana, Naoto Tamura, Shinsuke Yasuda, Keishi Fujio, Ayako Shoji, Hiroko Yamaguchi, Katsuhiko Iwasaki, Misako Makishima, Yuichi Kawata, Katsuhisa Yamashita, Ataru Igarashi
Summary: This study assessed the cost-effectiveness of biologic and targeted synthetic disease-modifying anti-rheumatic drugs (b/tsDMARDs) in the treatment of rheumatoid arthritis. The results showed that b/tsDMARDs with lower prices demonstrated higher cost-effectiveness.
MODERN RHEUMATOLOGY
(2023)
Article
Rheumatology
Yukiko Iwasaki, Yusuke Takeshima, Masahiro Nakano, Mai Okubo, Mineto Ota, Akari Suzuki, Yuta Kochi, Tomohisa Okamura, Takaho Endo, Ichiro Miki, Kazuhiro Sakurada, Kazuhiko Yamamoto, Keishi Fujio
Summary: This study explored the potential of plasma histidine levels as biomarkers for SLE patients. The results showed that histidine, along with other amino acids, could effectively distinguish between SLE patients and healthy controls. Furthermore, a decrease in histidine levels correlated with disease damage accrual, suggesting its involvement in SLE pathogenesis.
Article
Rheumatology
Saeko Yamada, Yasuo Nagafuchi, Min Wang, Mineto Ota, Hiroaki Hatano, Yusuke Takeshima, Mai Okubo, Satomi Kobayashi, Yusuke Sugimori, Nakano Masahiro, Ryochi Yoshida, Norio Hanata, Yuichi Suwa, Yumi Tsuchida, Yukiko Iwasaki, Shuji Sumitomo, Kanae Kubo, Kenichi Shimane, Keigo Setoguchi, Takanori Azuma, Hiroko Kanda, Hirofumi Shoda, Xuan Zhang, Kazuhiko Yamamoto, Kazuyoshi Ishigaki, Tomohisa Okamura, Keishi Fujio
Summary: By analyzing the transcriptome of peripheral blood immune cell subsets in patients with rheumatoid arthritis (RA), immune cells that predict treatment resistance were identified. Changes in the transcriptome and treatment effects in RA were characterized. Plasmacytoid dendritic cells (pDC) showed the strongest association with treatment resistance. The predictive value of pDC-related gene expression and pDC proportion in treatment-resistant patients was validated in independent cohorts.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Review
Biochemistry & Molecular Biology
Keishi Fujio
Summary: Systemic lupus erythematosus (SLE) is a complex autoimmune disease influenced by both genetic and environmental factors. Recent analysis has revealed the significance of the oxidative phosphorylation (OXPHOS) pathway in the pathogenesis of SLE, showing persistent activation in inactive SLE and its association with organ damage. The discovery that hydroxychloroquine (HCQ), which improves SLE prognosis, targets toll-like receptor (TLR) signaling upstream of OXPHOS highlights the clinical importance of this pathway. Further studies on OXPHOS-associated disease-susceptibility polymorphisms, gene expression, and protein function may aid in risk stratification of SLE.
Review
Medicine, General & Internal
Yumi Tsuchida, Hirofumi Shoda, Tetsuji Sawada, Keishi Fujio
Summary: Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease characterized by the production of autoantibodies and immune complex deposition. Autotaxin (ATX), which catalyzes the production of lysophosphatidic acid, has been identified as a potential biomarker in SLE. Increased levels of ATX have been found in the serum and urine of patients with SLE and lupus nephritis. Recent studies suggest that ATX produced by plasmacytoid dendritic cells may have an important role in SLE pathogenesis, and it is associated with type I interferons. ATX may be a valuable biomarker and key molecule in SLE.
FRONTIERS IN MEDICINE
(2023)
Article
Medicine, General & Internal
Hideki Oka, Shuji Sumitomo, Hayato Shimizu, Maki Kanamori, Shiori Murata, Daisuke Yamashita, Taiji Okada, Hiroaki Nishioka, Koichiro Ohmura
Summary: This case report reveals the relationship between anti-synthetase syndrome and pulmonary artery hypertension, as well as malignancy.
Letter
Rheumatology
Hideki Oka, Shuji Sumitomo, Masashi Nishikubo, Daisuke Yamashita, Hayato Maruoka, Yumi Shiroishi, Hayato Shimizu, Maki Kanamori, Hiroaki Nishioka, Koichiro Ohmura
Article
Rheumatology
Yusuke Sugimori, Yukiko Iwasaki, Yusuke Takeshima, Mai Okubo, Satomi Kobayashi, Hiroaki Hatano, Saeko Yamada, Masahiro Nakano, Ryochi Yoshida, Mineto Ota, Yumi Tsuchida, Yasuo Nagafuchi, Kenichi Shimane, Ken Yoshida, Daitaro Kurosaka, Shuji Sumitomo, Hirofumi Shoda, Kazuhiko Yamamoto, Tomohisa Okamura, Keishi Fujio
Summary: Transcriptome analysis identified common or specific immunological pathways in different immune cell types of patients with idiopathic inflammatory myopathies (IIM) based on their clinical phenotypes and myositis-specific antibodies (MSAs). B cells may play a significant role in both common and specific immunological pathways in IIM.
ACR OPEN RHEUMATOLOGY
(2023)
Letter
Rheumatology
T. Komai, T. Sawada, H. Tsuchiya, H. Harada, H. Shoda, K. Fujio
SCANDINAVIAN JOURNAL OF RHEUMATOLOGY
(2023)
Article
Multidisciplinary Sciences
Mariko Inoue, Yasuo Nagafuchi, Mineto Ota, Haruka Tsuchiya, Shoko Tateishi, Hiroko Kanda, Keishi Fujio
Summary: HLA-DRB1 * 04:05 allele is significantly associated with better disease improvement in abatacept-treated patients with rheumatoid arthritis. These disease-risk HLA alleles have the potential to serve as genomic biomarkers for predicting treatment response with co-stimulation blockage therapy.
SCIENTIFIC REPORTS
(2023)
Article
Rheumatology
Shinji Izuka, Toshihiko Komai, Hirofumi Shoda, Keishi Fujio
Summary: This study found that patients with myositis-specific antibody (MSA)-positive idiopathic inflammatory myopathy (IIM), specifically those with anti-MDA5 and anti-TIF1-gamma antibodies, have an increased long-term risk of malignancy. Screening for malignancies in patients with late phase of IIM and an anti-MDA5 antibody may be beneficial.
RHEUMATOLOGY INTERNATIONAL
(2023)
Article
Rheumatology
Daichi Umemoto, Shuji Sumitomo, Shohei Fujita, Hayato Shimizu, Hideki Oka, Maki Kanamori, Hiroaki Nishioka, Koichiro Ohmura
Summary: Patients with abnormally high anti-MDA-5 antibody titres may experience disease progression even if ferritin levels are not high and lung shadows are minimal. Therefore, early intensive treatment is necessary regardless of serum ferritin level.
MODERN RHEUMATOLOGY CASE REPORTS
(2023)
Article
Rheumatology
Hideki Oka, Shuji Sumitomo, Hayato Shimizu, Maki Kanamori, Daisuke Yamashita, Hiroaki Nishioka, Koichiro Ohmura
Summary: An 86-year-old man who had undergone endovascular aortic repair for an abdominal aortic aneurysm with impending rupture presented to the hospital due to fatigue and black stools. The diagnosis of aortic duodenal fistula was confirmed and emergency abdominal aortic replacement was performed. Pathological findings revealed the presence of numerous IgG4-positive plasma cells infiltrating the aortic wall, leading to a diagnosis of IgG4-related periaortitis.
MODERN RHEUMATOLOGY CASE REPORTS
(2023)